Risk of ADHD in children with childhood absence epilepsy versus controls: A population-based study.

Introduction: Children with childhood absence epilepsy (CAE) are deemed to be at higher risk of attention deficit hyperactivity disorder (ADHD), however the magnitude of that risk has not been assessed in a population-based study.

Methods: The Rochester Epidemiology Project database was used to identify children with a new diagnosis of CAE while resident in Olmsted County, MN between 1980-2018. For each case, four age- and sex-matched controls without epilepsy were identified.

Cenobamate in Generalized Epilepsy and Combined Generalized and Focal Epilepsy.

Background And Objectives: Cenobamate (CNB) is a United States Food and Drug Administration-approved antiseizure medication (ASM) for focal-onset seizures; however, its potential clinical effectiveness as a broad-spectrum ASM is not established. CNB has a proposed dual mechanism of action with preferential blockade of persistent sodium currents and positive allosteric modulation of the γ-aminobutyric acid-A (GABA-A) receptor. We evaluated the efficacy of CNB in drug refractory patients with genetic generalized epilepsies (GGE) or combined generalized and focal epilepsies (CGFE), including developmental and epileptic encephalopathies.

Temporal Encephalocele: A Treatable Etiology of Drug-Resistant Pediatric Temporal Lobe Epilepsy.

Background: Temporal lobe encephaloceles (TEs) are a rare cause of drug-resistant temporal lobe epilepsy (DR-TLE), with head trauma and obesity identified as risk factors in adults. This study evaluated the clinical characteristics of childhood-onset DR-TLE due to TE.

Methods: This is a single-institution retrospective review of childhood-onset DR-TLE with radiographic TE identified between 2008 and 2020.

Fenfluramine provides clinically meaningful reduction in frequency of drop seizures in patients with Lennox-Gastaut syndrome: Interim analysis of an open-label extension study.

Objective: This study was undertaken to evaluate the long-term safety and effectiveness of fenfluramine in patients with Lennox-Gastaut syndrome (LGS).

Methods: Eligible patients with LGS who completed a 14-week phase 3 randomized clinical trial enrolled in an open-label extension (OLE; NCT03355209). All patients were initially started on .

Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial.

Importance: New treatment options are needed for patients with Lennox-Gastaut syndrome (LGS), a profoundly impairing, treatment-resistant, developmental and epileptic encephalopathy.

Objective: To evaluate the efficacy and safety of fenfluramine in patients with LGS.

Design, Setting, And Participants: This multicenter, double-blind, placebo-controlled, parallel-group randomized clinical trial was conducted from November 27, 2017, to October 25, 2019, and had a 20-week trial duration.

Safety and efficacy of responsive neurostimulation in the pediatric population: Evidence from institutional review and patient-level meta-analysis.

Background: Responsive neurostimulation (RNS) is a novel technology for drug-resistant epilepsy rising from bilateral hemispheres or eloquent cortex. Although recently approved for adults, its safety and efficacy for pediatric patients is under investigation.

Methods: A comprehensive literature search (Pubmed/Medline, Scopus, Cochrane) was conducted for studies on RNS for pediatric epilepsy (<18 y/o) and supplemented by our institutional series (4 cases).

Masking for School-Age Children With Epilepsy: We Do Have Consensus!

Introduction: This study was designed to assess current recommendations from child neurologists and epileptologists on masking for school-age children with epilepsy.

Methods: A 7-item survey was created and sent out to members of the Child Neurology Society and Pediatric Epilepsy Research Consortium in August of 2021 to assess current practice and provider recommendations on masking.

Results: One hundred four individuals participated with representation from all regions of the United States.

Management of Infantile Spasms During the COVID-19 Pandemic.

Circumstances of the COVID-19 pandemic have mandated a change to standard management of infantile spasms. On April 6, 2020, the Child Neurology Society issued an online statement of immediate recommendations to streamline diagnosis and treatment of infantile spasms with utilization of telemedicine, outpatient studies, and selection of first-line oral therapies as initial treatment. The rationale for the recommendations and specific guidance including follow-up assessment are provided in this manuscript.

The Ketogenic and Modified Atkins Diet Therapy for Children With Refractory Epilepsy of Genetic Etiology.

Background: The ketogenic diet is an accepted treatment modality in refractory childhood epilepsy. In this study, we analyzed the efficacy and tolerability of the ketogenic and modified Atkins diets in children with refractory epilepsy of genetic etiology and studied the effect of the diet on seizure frequency.

Methods: The records of children with a genetic etiology for refractory epilepsy treated with ketogenic and modified Atkins diet between September 2005 and July 2016 were reviewed.

Jeavons Syndrome: Clinical Features and Response to Treatment.

Background: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized.

Methods: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016.

Impact of Prior Authorization of Antiepileptic Drugs in Children With Epilepsy.

Objective: We assessed how commonly prior authorization results in treatment delay or missed doses in children with epilepsy.

Methods: Parents of 462 children followed in a pediatric epilepsy clinic were surveyed regarding prior authorization in the preceding year. Epilepsy and insurance details were collected.

Cognitive and Social Outcomes of Epileptic Encephalopathies.

The term "epileptic encephalopathy" denotes a disorder in which seizures or frequent interictal discharges exacerbate neurocognitive dysfunction beyond what would be expected on the basis of underlying etiology. However, many underlying causes of epileptic encephalopathy also result in neurocognitive deficits, and it can be challenging to discern to what extent these deficits can be improved with better seizure control. Additionally, as seizures in these conditions are typically refractory, children are often exposed to high doses of multiple antiepileptic drugs which further exacerbate these comorbidities.

The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium.

Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia.

Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site.

Not all epileptic encephalopathies are Dravet syndrome: Early profound Thr226Met phenotype.

Objective: To define a distinct developmental and epileptic encephalopathy with early onset, profound impairment, and movement disorder.

Methods: A case series of 9 children were identified with a profound developmental and epileptic encephalopathy and mutation.

Results: We identified 9 children 3 to 12 years of age; 7 were male.

Predictive models in the diagnosis and treatment of autoimmune epilepsy.

Objective: To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy.

Methods: We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient.

Stiripentol in the Management of Epilepsy.

Stiripentol is a structurally unique antiepileptic drug that has several possible mechanisms of action, including diverse effects on the gamma-aminobutyric acid (GABA)- receptor and novel inhibition of lactate dehydrogenase. Because of its inhibition of several cytochrome P450 enzymes, it has extensive pharmacokinetic interactions, which often necessitates reduction in doses of certain co-therapies, particularly clobazam. Stiripentol also has a neuroprotective action, by reducing calcium-mediated neurotoxicity.

Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort.

Objective: Infantile spasms (IS) represent a severe epileptic encephalopathy presenting in the first 2 years of life. Recommended first-line therapies (hormonal therapy or vigabatrin) often fail. We evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome.

Usefulness of repeat review of head magnetic resonance images during presurgical epilepsy conferences.

Surgical epilepsy conferences are an important part of the process of determining whether a patient is a candidate for resective epilepsy surgery. At these conferences, repeat review (re-review) of the magnetic resonance images (MRIs) of the patient's head often occurs. This study assessed how often radiologic re-review at a presurgical epilepsy conference resulted in a changed interpretation of the head MRI.